Military Grooming Standards and Their Impact on Skin Diseases of the Head and Neck

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Military Grooming Standards and Their Impact on Skin Diseases of the Head and Neck

The US military enforces grooming standards to ensure the professional appearance and serviceability of soldiers in all operational settings. Although most individuals are able to uphold these regulations without incident, there is a growing cohort of servicemembers with skin diseases that were exacerbated or even initiated by haircuts, hairstyling, and shaving required to conform to these grooming standards. These skin diseases, which can affect both sexes and may not be appreciated until years into a soldier's service commitment, can have consequences related to individual morbidity and medical readiness for deployment, making it an important issue for medical practitioners to recognize and manage in servicemembers.

This review highlights several disorders of the pilosebaceous unit of the head and neck that can be caused or exacerbated by military grooming standards, including inflammatory hair disorders, traction alopecia, and pseudofolliculitis barbae. Discussion of each entity will include a review of susceptibility and causality as well as initial treatment options to consider (Table).

Inflammatory Hair Disorders

The proper appearance of servicemembers in uniform represents self-discipline and conformity to the high standards of the military. This transition occurs as a rite of passage for many new male recruits who receive shaved haircuts during their first days of basic training. Thereafter, male servicemembers are required to maintain a tapered appearance of the hair per military regulations.1 Clipping hair closely to the scalp or shaving the head entirely are authorized and often encouraged; therefore, high and tight haircuts and buzz cuts are popular among male soldiers due to the general ease of care and ability to maintain the haircut themselves. Conversely, these styles require servicemembers to get weekly or biweekly haircuts that in turn can lead to chronic trauma and irritation. In more susceptible populations, inflammatory hair disorders such as acne keloidalis nuchae (AKN), dissecting cellulitis of the scalp, and folliculitis decalvans may be incited.

Acne Keloidalis Nuchae
Acne keloidalis nuchae, also called folliculitis keloidalis, is a chronic scarring folliculitis presenting with papules and plaques on the occiput and nape of the neck that may merge to form hypertrophic scars or keloids. This disorder most commonly develops in young black men but also can be seen in black females and white patients of both sexes.2 Acne keloidalis nuchae shares many histologic features with central centrifugal cicatricial alopecia, which may suggest a similar pathogenesis. Apart from frequent haircuts, tight-collared shirts, such as those on military service uniforms, also have been associated with AKN. Because of these suspected etiologies, first-line treatment focuses on preventing further trauma by avoiding mechanical irritation and short haircuts, which may be difficult in the military setting. For earlier disease stages, topical and intralesional corticosteroids, oral retinoids, and topical and oral antibiotics are used for their anti-inflammatory properties.3 In refractory cases, surgical excision with healing by secondary intention may be attempted.4 Additional treatment options include the 1064-nm Nd:YAG and 810-nm diode lasers,3 UVB light therapy, CO2 laser, and radiotherapy.

Dissecting Cellulitis of the Scalp
Similar to AKN, dissecting cellulitis of the scalp is another inflammatory hair disorder that is worsened by frequent short haircuts.5 Dissecting cellulitis of the scalp is a primary cicatricial alopecia proposed to be secondary to follicular occlusion. It often is seen in black males aged 20 to 40 years and is characterized by boggy suppurative nodules and cysts with draining sinus tracts, abscesses, and resultant scarring alopecia. Dissecting cellulitis of the scalp is part of the follicular occlusion tetrad, which also includes hidradenitis suppurativa, acne conglobata, and pilonidal cysts. First-line therapies include topical and oral antibiotics, topical retinoids, intralesional corticosteroids, incision and drainage of fluctuant nodules, and oral isotretinoin with or without rifampin. Alternative treatments include oral zinc supplementation, oral corticosteroids, tumor necrosis factor α inhibitors, laser therapies, radiotherapy, and surgical management with wide local excision or total scalpectomy.6,7

Folliculitis Decalvans
Folliculitis decalvans is a primary cicatricial alopecia of the scalp that most commonly presents in middle-aged men without racial predilection.8 Folliculitis decalvans presents with multiple pustules, crusts, tufted hairs, and perifollicular hyperkeratosis, leading to scarring of the scalp, which often is most severe on the posterior vertex. Staphylococcus aureus is a presumed player in the pathogenesis of folliculitis decalvans with superantigens causing release of cytokines stimulating follicular destruction. Close haircuts in conformation with military grooming standards can contribute to this condition due to mechanical trauma and subsequent inflammation. It typically is diagnosed clinically, but if histologic confirmation is desired, a sample from the periphery of early lesions is preferred.9 Initial treatment consists of antibacterial shampoos, topical corticosteroids, topical antibiotics, and combination oral antibiotic therapy with rifampin and clindamycin. Studies using oral isotretinoin have shown variable results,10,11 and the most effective treatment of recalcitrant lesions appears to be intralesional corticosteroids.12

Follicular and Scarring Disorders

In addition to inflammatory hair disorders, military grooming standards have been linked to the pathogenesis of diseases such as pseudofolliculitis barbae, traction alopecia, and keloids, specifically through irritation of the face, neck, and scalp, as well as damage to the follicular unit.5 These conditions develop because grooming regulations necessitate certain hair practices such as close shaving of facial and neck hair and keeping long hair secured relatively tightly to the scalp.

Pseudofolliculitis Barbae
Males in the military are obligated to keep their faces clean-shaven.1 They may acquire a medical waiver for a specified beard length if deemed appropriate by the treating physician,1 which often leads to the need for continual waiver renewal and also may warrant possible negative perception from peers, subordinates, and leadership. One of the most prevalent conditions that is closely associated with shaving is pseudofolliculitis barbae. The combination of close shaving and tightly coiled hairs causes the hairs to grow toward and penetrate the skin, particularly on the neck.13 In some cases, the hairs never actually exit the skin and simply curl within the superficial epidermis. A foreign body reaction often arises, leading to inflamed follicular papules and pustules. Affected individuals may experience pain, pruritus, and secondary infections. Postinflammatory hyperpigmentation, hypertrophic scarring, and keloid formation are common sequelae in cases of untreated disease. Pseudofolliculitis barbae also is exacerbated by pulling the skin taut and shaving against the grain, making behavioral interventions a key component in management of this condition. Preliminary recommendations include using a new or electric razor, leaving hair at least 2 mm in length, and shaving in the direction of hair growth. Other treatment options with varying effectiveness include daily alternation of a mild topical corticosteroid and one of the following: a topical retinoid, topical antibiotics, or glycolic acid. The only treatments that approach definitive cure are laser hair removal and electrolysis for which patient skin type plays an important role in laser selection.5

Traction Alopecia
Similar to their male counterparts, female military members must also present a conservative professional appearance, including hair that is neatly groomed.1 If the length of the hair extends beyond the uniform collar, it must be inconspicuously fastened or pinned above the collar. As a result, loosely tied hair is unauthorized, and females with long hair must secure their hair tightly on a daily basis. Traction alopecia results from tight hairstyling over a prolonged period and commonly affects female soldiers. The etiology is presumed to be mechanical loosening of hair within the follicles, leading to inflammation. Although traditionally seen in black women along the frontal and temporal hairlines, traction alopecia has been identified in individuals of all races and can occur anywhere on the scalp.5 Perifollicular erythema may be the first sign, and papules and pustules may be visible. Although the hair loss in traction alopecia usually is reversible if the traction is ceased, end-stage disease may be permanent.6 Halting traction-inducing practices is paramount, and other treatment options that may slow progression include topical or oral antibiotics and topical or intralesional corticosteroids. Recovery of hair loss also may be aided by topical minoxidil.5

Keloids
Keloid formation is an important pathology to address, as it may result from several of the aforementioned conditions. Keloids are most commonly seen in black individuals but also can occur in Hispanic and Asian patients. The cause has not been fully elucidated but is thought to be a combination of dysfunctional fibroblasts with a genetic component based on racial predilection and twin concordance studies.5 The chest, shoulders, upper back, neck, and earlobes are particularly susceptible to keloid formation, which can appear from 1 to 24 years following dermal trauma.5 Unlike hypertrophic scars, keloids generally do not regress and frequently cause discomfort, pruritus, and emotional distress. They also can hinder wearing a military uniform. Sustained remission is problematic, making prevention a first-line approach, including proper care of wounds when they occur and avoiding elective procedures such as piercings and tattoos. Intralesional corticosteroids, adjuvant injections (eg, 5-fluorouracil), silicone sheeting, cryotherapy, radiation, laser therapy, and excision are some of the treatment options when keloids have formed.5

Final Comment

It is important to recognize military grooming standards as a cause or contributor to several diseases of the head and neck in military servicemembers. Specifically, frequent haircuts in male soldiers are associated with several inflammatory hair disorders, including AKN, dissecting cellulitis of the scalp, and folliculitis decalvans, while daily shaving predisposes individuals to pseudofolliculitis barbae with possible keloid formation. Females may develop traction alopecia from chronically tight, pulled back hairstyles. All of these conditions have health implications for the affected individuals and can compromise the military mission. Awareness, prevention, and recognition are key along with the knowledge base to provide anticipatory avoidance and initiate appropriate treatments, thereby mitigating these potential consequences.

References
  1. US Department of the Army. Wear and Appearance of Army Uniforms and Insignia: Army Regulation 670-1. Washington, DC: Department of the Army; 2017. https://history.army.mil/html/forcestruc/docs/AR670-1.pdf. Accessed October 11, 2018.
  2. East-Innis AD, Stylianou K, Paolino A, et al. Acne keloidalis nuchae: risk factors and associated disorders--a retrospective study. Int J Dermatol. 2017;56:828-832.
  3. Maranda EL, Simmons BJ, Nguyen AH, et al. Treatment of acne keloidalis nuchae: a systematic review of the literature. Dermatol Ther (Heidelb). 2016;6:363-378.
  4. Glenn MJ, Bennett RG, Kelly AP. Acne keloidalis nuchae: treatment with excision and second-intention healing. J Am Acad Dermatol. 1995;33:243-246.
  5. Madu P, Kundu RV. Follicular and scarring disorders in skin of color: presentation and management. Am J Clin Dermatol. 2014;15:307-321.
  6. Rodney IJ, Onwudiwe OC. Hair and scalp disorders in ethnic populations. J Drugs Dermatol. 2013;12:420-427.
  7. Lindsey SF, Tosti A. Ethnic hair disorders. Curr Probl Dermatol. 2015;47:139-148.
  8. Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin Dermatol. 2001;19:211-225.
  9. Sperling LC, Cowper SE, Knopp EA. An Atlas of Hair Pathology with Clinical Correlations. 2nd ed. Boca Raton, FL: CRC Press; 2012.
  10. Gemmeke A, Wollina U. Folliculitis decalvans of the scalp: response to triple therapy with isotretinoin, clindamycin, and prednisolone. Acta Dermatovenerol Alp Pannonica Adriat. 2006;15:184-186.
  11. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  12. Bolduc C, Sperling LC, Shapiro J. Primary cicatricial alopecia. J Am Acad Dermatol. 2016;75:101-117.
  13. Perry PK, Cook-Bolden FE, Rahman Z, et al. Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. J Am Acad Dermatol. 2002;46(2 suppl):S113-S119.
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Ms. Weiss is from Uniformed Services University, Bethesda, Maryland. Drs. Arballo, Miletta, and Wohltmann are from San Antonio Uniformed Services Health Education Consortium, Joint Base San Antonio, Texas.

The authors report no conflict of interest.

The opinions and assertions expressed herein are those of the authors and do not reflect the official policy or position of San Antonio Military Medical Center, Uniformed Services University, the Department of the Army, the Department of the Air Force, or the Department of Defense.

Correspondence: Wendi E. Wohltmann, MD, Department of Dermatology, 1100 Wilford Hall Loop, Bldg 4554, JBSA Lackland, TX 78236 ([email protected]).

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Author and Disclosure Information

Ms. Weiss is from Uniformed Services University, Bethesda, Maryland. Drs. Arballo, Miletta, and Wohltmann are from San Antonio Uniformed Services Health Education Consortium, Joint Base San Antonio, Texas.

The authors report no conflict of interest.

The opinions and assertions expressed herein are those of the authors and do not reflect the official policy or position of San Antonio Military Medical Center, Uniformed Services University, the Department of the Army, the Department of the Air Force, or the Department of Defense.

Correspondence: Wendi E. Wohltmann, MD, Department of Dermatology, 1100 Wilford Hall Loop, Bldg 4554, JBSA Lackland, TX 78236 ([email protected]).

Author and Disclosure Information

Ms. Weiss is from Uniformed Services University, Bethesda, Maryland. Drs. Arballo, Miletta, and Wohltmann are from San Antonio Uniformed Services Health Education Consortium, Joint Base San Antonio, Texas.

The authors report no conflict of interest.

The opinions and assertions expressed herein are those of the authors and do not reflect the official policy or position of San Antonio Military Medical Center, Uniformed Services University, the Department of the Army, the Department of the Air Force, or the Department of Defense.

Correspondence: Wendi E. Wohltmann, MD, Department of Dermatology, 1100 Wilford Hall Loop, Bldg 4554, JBSA Lackland, TX 78236 ([email protected]).

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The US military enforces grooming standards to ensure the professional appearance and serviceability of soldiers in all operational settings. Although most individuals are able to uphold these regulations without incident, there is a growing cohort of servicemembers with skin diseases that were exacerbated or even initiated by haircuts, hairstyling, and shaving required to conform to these grooming standards. These skin diseases, which can affect both sexes and may not be appreciated until years into a soldier's service commitment, can have consequences related to individual morbidity and medical readiness for deployment, making it an important issue for medical practitioners to recognize and manage in servicemembers.

This review highlights several disorders of the pilosebaceous unit of the head and neck that can be caused or exacerbated by military grooming standards, including inflammatory hair disorders, traction alopecia, and pseudofolliculitis barbae. Discussion of each entity will include a review of susceptibility and causality as well as initial treatment options to consider (Table).

Inflammatory Hair Disorders

The proper appearance of servicemembers in uniform represents self-discipline and conformity to the high standards of the military. This transition occurs as a rite of passage for many new male recruits who receive shaved haircuts during their first days of basic training. Thereafter, male servicemembers are required to maintain a tapered appearance of the hair per military regulations.1 Clipping hair closely to the scalp or shaving the head entirely are authorized and often encouraged; therefore, high and tight haircuts and buzz cuts are popular among male soldiers due to the general ease of care and ability to maintain the haircut themselves. Conversely, these styles require servicemembers to get weekly or biweekly haircuts that in turn can lead to chronic trauma and irritation. In more susceptible populations, inflammatory hair disorders such as acne keloidalis nuchae (AKN), dissecting cellulitis of the scalp, and folliculitis decalvans may be incited.

Acne Keloidalis Nuchae
Acne keloidalis nuchae, also called folliculitis keloidalis, is a chronic scarring folliculitis presenting with papules and plaques on the occiput and nape of the neck that may merge to form hypertrophic scars or keloids. This disorder most commonly develops in young black men but also can be seen in black females and white patients of both sexes.2 Acne keloidalis nuchae shares many histologic features with central centrifugal cicatricial alopecia, which may suggest a similar pathogenesis. Apart from frequent haircuts, tight-collared shirts, such as those on military service uniforms, also have been associated with AKN. Because of these suspected etiologies, first-line treatment focuses on preventing further trauma by avoiding mechanical irritation and short haircuts, which may be difficult in the military setting. For earlier disease stages, topical and intralesional corticosteroids, oral retinoids, and topical and oral antibiotics are used for their anti-inflammatory properties.3 In refractory cases, surgical excision with healing by secondary intention may be attempted.4 Additional treatment options include the 1064-nm Nd:YAG and 810-nm diode lasers,3 UVB light therapy, CO2 laser, and radiotherapy.

Dissecting Cellulitis of the Scalp
Similar to AKN, dissecting cellulitis of the scalp is another inflammatory hair disorder that is worsened by frequent short haircuts.5 Dissecting cellulitis of the scalp is a primary cicatricial alopecia proposed to be secondary to follicular occlusion. It often is seen in black males aged 20 to 40 years and is characterized by boggy suppurative nodules and cysts with draining sinus tracts, abscesses, and resultant scarring alopecia. Dissecting cellulitis of the scalp is part of the follicular occlusion tetrad, which also includes hidradenitis suppurativa, acne conglobata, and pilonidal cysts. First-line therapies include topical and oral antibiotics, topical retinoids, intralesional corticosteroids, incision and drainage of fluctuant nodules, and oral isotretinoin with or without rifampin. Alternative treatments include oral zinc supplementation, oral corticosteroids, tumor necrosis factor α inhibitors, laser therapies, radiotherapy, and surgical management with wide local excision or total scalpectomy.6,7

Folliculitis Decalvans
Folliculitis decalvans is a primary cicatricial alopecia of the scalp that most commonly presents in middle-aged men without racial predilection.8 Folliculitis decalvans presents with multiple pustules, crusts, tufted hairs, and perifollicular hyperkeratosis, leading to scarring of the scalp, which often is most severe on the posterior vertex. Staphylococcus aureus is a presumed player in the pathogenesis of folliculitis decalvans with superantigens causing release of cytokines stimulating follicular destruction. Close haircuts in conformation with military grooming standards can contribute to this condition due to mechanical trauma and subsequent inflammation. It typically is diagnosed clinically, but if histologic confirmation is desired, a sample from the periphery of early lesions is preferred.9 Initial treatment consists of antibacterial shampoos, topical corticosteroids, topical antibiotics, and combination oral antibiotic therapy with rifampin and clindamycin. Studies using oral isotretinoin have shown variable results,10,11 and the most effective treatment of recalcitrant lesions appears to be intralesional corticosteroids.12

Follicular and Scarring Disorders

In addition to inflammatory hair disorders, military grooming standards have been linked to the pathogenesis of diseases such as pseudofolliculitis barbae, traction alopecia, and keloids, specifically through irritation of the face, neck, and scalp, as well as damage to the follicular unit.5 These conditions develop because grooming regulations necessitate certain hair practices such as close shaving of facial and neck hair and keeping long hair secured relatively tightly to the scalp.

Pseudofolliculitis Barbae
Males in the military are obligated to keep their faces clean-shaven.1 They may acquire a medical waiver for a specified beard length if deemed appropriate by the treating physician,1 which often leads to the need for continual waiver renewal and also may warrant possible negative perception from peers, subordinates, and leadership. One of the most prevalent conditions that is closely associated with shaving is pseudofolliculitis barbae. The combination of close shaving and tightly coiled hairs causes the hairs to grow toward and penetrate the skin, particularly on the neck.13 In some cases, the hairs never actually exit the skin and simply curl within the superficial epidermis. A foreign body reaction often arises, leading to inflamed follicular papules and pustules. Affected individuals may experience pain, pruritus, and secondary infections. Postinflammatory hyperpigmentation, hypertrophic scarring, and keloid formation are common sequelae in cases of untreated disease. Pseudofolliculitis barbae also is exacerbated by pulling the skin taut and shaving against the grain, making behavioral interventions a key component in management of this condition. Preliminary recommendations include using a new or electric razor, leaving hair at least 2 mm in length, and shaving in the direction of hair growth. Other treatment options with varying effectiveness include daily alternation of a mild topical corticosteroid and one of the following: a topical retinoid, topical antibiotics, or glycolic acid. The only treatments that approach definitive cure are laser hair removal and electrolysis for which patient skin type plays an important role in laser selection.5

Traction Alopecia
Similar to their male counterparts, female military members must also present a conservative professional appearance, including hair that is neatly groomed.1 If the length of the hair extends beyond the uniform collar, it must be inconspicuously fastened or pinned above the collar. As a result, loosely tied hair is unauthorized, and females with long hair must secure their hair tightly on a daily basis. Traction alopecia results from tight hairstyling over a prolonged period and commonly affects female soldiers. The etiology is presumed to be mechanical loosening of hair within the follicles, leading to inflammation. Although traditionally seen in black women along the frontal and temporal hairlines, traction alopecia has been identified in individuals of all races and can occur anywhere on the scalp.5 Perifollicular erythema may be the first sign, and papules and pustules may be visible. Although the hair loss in traction alopecia usually is reversible if the traction is ceased, end-stage disease may be permanent.6 Halting traction-inducing practices is paramount, and other treatment options that may slow progression include topical or oral antibiotics and topical or intralesional corticosteroids. Recovery of hair loss also may be aided by topical minoxidil.5

Keloids
Keloid formation is an important pathology to address, as it may result from several of the aforementioned conditions. Keloids are most commonly seen in black individuals but also can occur in Hispanic and Asian patients. The cause has not been fully elucidated but is thought to be a combination of dysfunctional fibroblasts with a genetic component based on racial predilection and twin concordance studies.5 The chest, shoulders, upper back, neck, and earlobes are particularly susceptible to keloid formation, which can appear from 1 to 24 years following dermal trauma.5 Unlike hypertrophic scars, keloids generally do not regress and frequently cause discomfort, pruritus, and emotional distress. They also can hinder wearing a military uniform. Sustained remission is problematic, making prevention a first-line approach, including proper care of wounds when they occur and avoiding elective procedures such as piercings and tattoos. Intralesional corticosteroids, adjuvant injections (eg, 5-fluorouracil), silicone sheeting, cryotherapy, radiation, laser therapy, and excision are some of the treatment options when keloids have formed.5

Final Comment

It is important to recognize military grooming standards as a cause or contributor to several diseases of the head and neck in military servicemembers. Specifically, frequent haircuts in male soldiers are associated with several inflammatory hair disorders, including AKN, dissecting cellulitis of the scalp, and folliculitis decalvans, while daily shaving predisposes individuals to pseudofolliculitis barbae with possible keloid formation. Females may develop traction alopecia from chronically tight, pulled back hairstyles. All of these conditions have health implications for the affected individuals and can compromise the military mission. Awareness, prevention, and recognition are key along with the knowledge base to provide anticipatory avoidance and initiate appropriate treatments, thereby mitigating these potential consequences.

The US military enforces grooming standards to ensure the professional appearance and serviceability of soldiers in all operational settings. Although most individuals are able to uphold these regulations without incident, there is a growing cohort of servicemembers with skin diseases that were exacerbated or even initiated by haircuts, hairstyling, and shaving required to conform to these grooming standards. These skin diseases, which can affect both sexes and may not be appreciated until years into a soldier's service commitment, can have consequences related to individual morbidity and medical readiness for deployment, making it an important issue for medical practitioners to recognize and manage in servicemembers.

This review highlights several disorders of the pilosebaceous unit of the head and neck that can be caused or exacerbated by military grooming standards, including inflammatory hair disorders, traction alopecia, and pseudofolliculitis barbae. Discussion of each entity will include a review of susceptibility and causality as well as initial treatment options to consider (Table).

Inflammatory Hair Disorders

The proper appearance of servicemembers in uniform represents self-discipline and conformity to the high standards of the military. This transition occurs as a rite of passage for many new male recruits who receive shaved haircuts during their first days of basic training. Thereafter, male servicemembers are required to maintain a tapered appearance of the hair per military regulations.1 Clipping hair closely to the scalp or shaving the head entirely are authorized and often encouraged; therefore, high and tight haircuts and buzz cuts are popular among male soldiers due to the general ease of care and ability to maintain the haircut themselves. Conversely, these styles require servicemembers to get weekly or biweekly haircuts that in turn can lead to chronic trauma and irritation. In more susceptible populations, inflammatory hair disorders such as acne keloidalis nuchae (AKN), dissecting cellulitis of the scalp, and folliculitis decalvans may be incited.

Acne Keloidalis Nuchae
Acne keloidalis nuchae, also called folliculitis keloidalis, is a chronic scarring folliculitis presenting with papules and plaques on the occiput and nape of the neck that may merge to form hypertrophic scars or keloids. This disorder most commonly develops in young black men but also can be seen in black females and white patients of both sexes.2 Acne keloidalis nuchae shares many histologic features with central centrifugal cicatricial alopecia, which may suggest a similar pathogenesis. Apart from frequent haircuts, tight-collared shirts, such as those on military service uniforms, also have been associated with AKN. Because of these suspected etiologies, first-line treatment focuses on preventing further trauma by avoiding mechanical irritation and short haircuts, which may be difficult in the military setting. For earlier disease stages, topical and intralesional corticosteroids, oral retinoids, and topical and oral antibiotics are used for their anti-inflammatory properties.3 In refractory cases, surgical excision with healing by secondary intention may be attempted.4 Additional treatment options include the 1064-nm Nd:YAG and 810-nm diode lasers,3 UVB light therapy, CO2 laser, and radiotherapy.

Dissecting Cellulitis of the Scalp
Similar to AKN, dissecting cellulitis of the scalp is another inflammatory hair disorder that is worsened by frequent short haircuts.5 Dissecting cellulitis of the scalp is a primary cicatricial alopecia proposed to be secondary to follicular occlusion. It often is seen in black males aged 20 to 40 years and is characterized by boggy suppurative nodules and cysts with draining sinus tracts, abscesses, and resultant scarring alopecia. Dissecting cellulitis of the scalp is part of the follicular occlusion tetrad, which also includes hidradenitis suppurativa, acne conglobata, and pilonidal cysts. First-line therapies include topical and oral antibiotics, topical retinoids, intralesional corticosteroids, incision and drainage of fluctuant nodules, and oral isotretinoin with or without rifampin. Alternative treatments include oral zinc supplementation, oral corticosteroids, tumor necrosis factor α inhibitors, laser therapies, radiotherapy, and surgical management with wide local excision or total scalpectomy.6,7

Folliculitis Decalvans
Folliculitis decalvans is a primary cicatricial alopecia of the scalp that most commonly presents in middle-aged men without racial predilection.8 Folliculitis decalvans presents with multiple pustules, crusts, tufted hairs, and perifollicular hyperkeratosis, leading to scarring of the scalp, which often is most severe on the posterior vertex. Staphylococcus aureus is a presumed player in the pathogenesis of folliculitis decalvans with superantigens causing release of cytokines stimulating follicular destruction. Close haircuts in conformation with military grooming standards can contribute to this condition due to mechanical trauma and subsequent inflammation. It typically is diagnosed clinically, but if histologic confirmation is desired, a sample from the periphery of early lesions is preferred.9 Initial treatment consists of antibacterial shampoos, topical corticosteroids, topical antibiotics, and combination oral antibiotic therapy with rifampin and clindamycin. Studies using oral isotretinoin have shown variable results,10,11 and the most effective treatment of recalcitrant lesions appears to be intralesional corticosteroids.12

Follicular and Scarring Disorders

In addition to inflammatory hair disorders, military grooming standards have been linked to the pathogenesis of diseases such as pseudofolliculitis barbae, traction alopecia, and keloids, specifically through irritation of the face, neck, and scalp, as well as damage to the follicular unit.5 These conditions develop because grooming regulations necessitate certain hair practices such as close shaving of facial and neck hair and keeping long hair secured relatively tightly to the scalp.

Pseudofolliculitis Barbae
Males in the military are obligated to keep their faces clean-shaven.1 They may acquire a medical waiver for a specified beard length if deemed appropriate by the treating physician,1 which often leads to the need for continual waiver renewal and also may warrant possible negative perception from peers, subordinates, and leadership. One of the most prevalent conditions that is closely associated with shaving is pseudofolliculitis barbae. The combination of close shaving and tightly coiled hairs causes the hairs to grow toward and penetrate the skin, particularly on the neck.13 In some cases, the hairs never actually exit the skin and simply curl within the superficial epidermis. A foreign body reaction often arises, leading to inflamed follicular papules and pustules. Affected individuals may experience pain, pruritus, and secondary infections. Postinflammatory hyperpigmentation, hypertrophic scarring, and keloid formation are common sequelae in cases of untreated disease. Pseudofolliculitis barbae also is exacerbated by pulling the skin taut and shaving against the grain, making behavioral interventions a key component in management of this condition. Preliminary recommendations include using a new or electric razor, leaving hair at least 2 mm in length, and shaving in the direction of hair growth. Other treatment options with varying effectiveness include daily alternation of a mild topical corticosteroid and one of the following: a topical retinoid, topical antibiotics, or glycolic acid. The only treatments that approach definitive cure are laser hair removal and electrolysis for which patient skin type plays an important role in laser selection.5

Traction Alopecia
Similar to their male counterparts, female military members must also present a conservative professional appearance, including hair that is neatly groomed.1 If the length of the hair extends beyond the uniform collar, it must be inconspicuously fastened or pinned above the collar. As a result, loosely tied hair is unauthorized, and females with long hair must secure their hair tightly on a daily basis. Traction alopecia results from tight hairstyling over a prolonged period and commonly affects female soldiers. The etiology is presumed to be mechanical loosening of hair within the follicles, leading to inflammation. Although traditionally seen in black women along the frontal and temporal hairlines, traction alopecia has been identified in individuals of all races and can occur anywhere on the scalp.5 Perifollicular erythema may be the first sign, and papules and pustules may be visible. Although the hair loss in traction alopecia usually is reversible if the traction is ceased, end-stage disease may be permanent.6 Halting traction-inducing practices is paramount, and other treatment options that may slow progression include topical or oral antibiotics and topical or intralesional corticosteroids. Recovery of hair loss also may be aided by topical minoxidil.5

Keloids
Keloid formation is an important pathology to address, as it may result from several of the aforementioned conditions. Keloids are most commonly seen in black individuals but also can occur in Hispanic and Asian patients. The cause has not been fully elucidated but is thought to be a combination of dysfunctional fibroblasts with a genetic component based on racial predilection and twin concordance studies.5 The chest, shoulders, upper back, neck, and earlobes are particularly susceptible to keloid formation, which can appear from 1 to 24 years following dermal trauma.5 Unlike hypertrophic scars, keloids generally do not regress and frequently cause discomfort, pruritus, and emotional distress. They also can hinder wearing a military uniform. Sustained remission is problematic, making prevention a first-line approach, including proper care of wounds when they occur and avoiding elective procedures such as piercings and tattoos. Intralesional corticosteroids, adjuvant injections (eg, 5-fluorouracil), silicone sheeting, cryotherapy, radiation, laser therapy, and excision are some of the treatment options when keloids have formed.5

Final Comment

It is important to recognize military grooming standards as a cause or contributor to several diseases of the head and neck in military servicemembers. Specifically, frequent haircuts in male soldiers are associated with several inflammatory hair disorders, including AKN, dissecting cellulitis of the scalp, and folliculitis decalvans, while daily shaving predisposes individuals to pseudofolliculitis barbae with possible keloid formation. Females may develop traction alopecia from chronically tight, pulled back hairstyles. All of these conditions have health implications for the affected individuals and can compromise the military mission. Awareness, prevention, and recognition are key along with the knowledge base to provide anticipatory avoidance and initiate appropriate treatments, thereby mitigating these potential consequences.

References
  1. US Department of the Army. Wear and Appearance of Army Uniforms and Insignia: Army Regulation 670-1. Washington, DC: Department of the Army; 2017. https://history.army.mil/html/forcestruc/docs/AR670-1.pdf. Accessed October 11, 2018.
  2. East-Innis AD, Stylianou K, Paolino A, et al. Acne keloidalis nuchae: risk factors and associated disorders--a retrospective study. Int J Dermatol. 2017;56:828-832.
  3. Maranda EL, Simmons BJ, Nguyen AH, et al. Treatment of acne keloidalis nuchae: a systematic review of the literature. Dermatol Ther (Heidelb). 2016;6:363-378.
  4. Glenn MJ, Bennett RG, Kelly AP. Acne keloidalis nuchae: treatment with excision and second-intention healing. J Am Acad Dermatol. 1995;33:243-246.
  5. Madu P, Kundu RV. Follicular and scarring disorders in skin of color: presentation and management. Am J Clin Dermatol. 2014;15:307-321.
  6. Rodney IJ, Onwudiwe OC. Hair and scalp disorders in ethnic populations. J Drugs Dermatol. 2013;12:420-427.
  7. Lindsey SF, Tosti A. Ethnic hair disorders. Curr Probl Dermatol. 2015;47:139-148.
  8. Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin Dermatol. 2001;19:211-225.
  9. Sperling LC, Cowper SE, Knopp EA. An Atlas of Hair Pathology with Clinical Correlations. 2nd ed. Boca Raton, FL: CRC Press; 2012.
  10. Gemmeke A, Wollina U. Folliculitis decalvans of the scalp: response to triple therapy with isotretinoin, clindamycin, and prednisolone. Acta Dermatovenerol Alp Pannonica Adriat. 2006;15:184-186.
  11. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  12. Bolduc C, Sperling LC, Shapiro J. Primary cicatricial alopecia. J Am Acad Dermatol. 2016;75:101-117.
  13. Perry PK, Cook-Bolden FE, Rahman Z, et al. Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. J Am Acad Dermatol. 2002;46(2 suppl):S113-S119.
References
  1. US Department of the Army. Wear and Appearance of Army Uniforms and Insignia: Army Regulation 670-1. Washington, DC: Department of the Army; 2017. https://history.army.mil/html/forcestruc/docs/AR670-1.pdf. Accessed October 11, 2018.
  2. East-Innis AD, Stylianou K, Paolino A, et al. Acne keloidalis nuchae: risk factors and associated disorders--a retrospective study. Int J Dermatol. 2017;56:828-832.
  3. Maranda EL, Simmons BJ, Nguyen AH, et al. Treatment of acne keloidalis nuchae: a systematic review of the literature. Dermatol Ther (Heidelb). 2016;6:363-378.
  4. Glenn MJ, Bennett RG, Kelly AP. Acne keloidalis nuchae: treatment with excision and second-intention healing. J Am Acad Dermatol. 1995;33:243-246.
  5. Madu P, Kundu RV. Follicular and scarring disorders in skin of color: presentation and management. Am J Clin Dermatol. 2014;15:307-321.
  6. Rodney IJ, Onwudiwe OC. Hair and scalp disorders in ethnic populations. J Drugs Dermatol. 2013;12:420-427.
  7. Lindsey SF, Tosti A. Ethnic hair disorders. Curr Probl Dermatol. 2015;47:139-148.
  8. Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin Dermatol. 2001;19:211-225.
  9. Sperling LC, Cowper SE, Knopp EA. An Atlas of Hair Pathology with Clinical Correlations. 2nd ed. Boca Raton, FL: CRC Press; 2012.
  10. Gemmeke A, Wollina U. Folliculitis decalvans of the scalp: response to triple therapy with isotretinoin, clindamycin, and prednisolone. Acta Dermatovenerol Alp Pannonica Adriat. 2006;15:184-186.
  11. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  12. Bolduc C, Sperling LC, Shapiro J. Primary cicatricial alopecia. J Am Acad Dermatol. 2016;75:101-117.
  13. Perry PK, Cook-Bolden FE, Rahman Z, et al. Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. J Am Acad Dermatol. 2002;46(2 suppl):S113-S119.
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Practice Points

  • The short frequent haircuts required to maintain a tapered appearance of the hair per US military regulations may lead to inflammatory hair disorders such as acne keloidalis nuchae, dissecting cellulitis of the scalp, and folliculitis decalvans.
  • The mainstay of prevention for these conditions is avoidance of inciting factors such as short haircuts, tight-collared shirts, frequent shaving, or tight hairstyles.
  • Early identification and treatment of inflammatory follicular and scarring disorders can prevent further scarring, pigmentation changes, and/or disfigurement.
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Enlarging Breast Lesion

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The Diagnosis: Radiation-Associated Angiosarcoma

At the time of presentation, a 4-mm lesional punch biopsy was obtained (Figure), which revealed an epithelioid neoplasm within the dermis expressing CD31 and CD34, and staining negatively for S-100, CD45, and estrogen and progesterone receptors. The histologic and immunophenotypic findings were compatible with the diagnosis of angiosarcoma. Given the patient’s history of radiation for breast carcinoma several years ago, this tumor was consistent with radiation-associated angiosarcoma (RAAS).

Findings from a lesional punch biopsy were consistent with angiosarcoma (A and B)(H&E, original magnification ×40 and ×400, respectively).

Development of secondary angiosarcoma has been linked to both prior radiation (RAAS) and chronic lymphedema (Stewart-Treves syndrome).1 Radiation-associated angiosarcoma is defined as a “pathologically confirmed breast or chest wall angiosarcoma arising within a previously irradiated field.”2 The incidence of RAAS is estimated to be 0.9 per 1000 individuals following radiation treatment of breast cancer over the subsequent 15 years and a mean time from radiation to development of 7 years.1 Incidence is expected to increase in the future due to improved likelihood of surviving early-stage breast carcinoma and the increased use of external beam radiation therapy for management of breast cancer.

Differentiating between primary and secondary angiosarcoma of the breast is important. Although primary breast angiosarcoma usually arises in women aged 30 to 40 years, RAAS tends to arise in older women (mean age, 68 years) and is seen only in those women with prior radiation.2 Additionally, high-level amplification of MYC, a known photo-oncogene, on chromosome 8 is a key genetic alteration of RAAS that helps to distinguish it from primary angiosarcoma, though this variance may be present in only half of RAAS cases.3 Immunohistochemical analysis of tumor cells for MYC expression correlates well with this amplification and also is helpful in distinguishing atypical vascular lesions from RAAS.4 Atypical vascular lesions, similar to RAAS, occur years after radiation exposure and may have a similar clinical presentation. Atypical vascular lesions do not progress to angiosarcoma in reported cases, but clinical and histologic overlap with RAAS make the diagnosis difficult.5 In these cases, analysis with fluorescence in situ hybridization or immunohistochemistry for the MYC amplification is important to differentiate these tumors.6

At the time of presentation, the majority of patients with RAAS of the breast have localized disease, often with a variable presentation. In all known cases, there have been skin changes present, emphasizing the importance of both patient and clinician vigilance on a regular basis in at-risk individuals. In one study, the most common presentation was breast ecchymosis, which was observed in 55% of patients.7 These lesions involve the dermis and are commonly mistaken for benign conditions such as infection or hemorrhage.2 In 2 other studies, RAAS most often manifested as a skin nodule or apparent tumor, closely followed by either a rash or bruiselike presentation.1,2

The overall recommendation for management of patients with ecchymotic skin lesions in previously irradiated regions is to obtain a biopsy specimen for tissue diagnosis. Although there is no standard of care for the management of RAAS, a multidisciplinary approach involving specialists from oncology, surgical oncology, and radiation oncology is recommended. Most often, radical surgery encompassing both the breast parenchyma and the at-risk radiated skin is performed. Extensive surgery has demonstrated the best survival benefits compared to mastectomy alone.7 Chemotherapeutics also may be used as adjuncts to surgery, which have been determined to decrease local recurrence rates but have no proven survival benefits.2 Adverse prognostic factors for survival are tumor size greater than 10 cm and development of local and/or distant metastases.2 Following the diagnosis of RAAS, our patient underwent radical mastectomy with adjuvant chemotherapy and remained disease free 6 months after surgery.

In summary, RAAS is a well-known, albeit relatively uncommon, consequence of radiation therapy. Dermatologists, oncologists, and primary care providers play an important role in recognizing this entity when evaluating patients with ecchymotic lesions as well as nodules or tumors within an irradiated field. Biopsy should be obtained promptly to prevent delay in diagnosis and to expedite referral to appropriate specialists for further evaluation and treatment.

References
  1. Seinen JM, Emelie S, Verstappen V, et al. Radiation-associated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection. Ann Surg Oncol. 2012;19:2700-2706.
  2. Torres KE, Ravi V, Kin K, et al. Long-term outcomes in patients with radiation-associated angiosarcomas of the breast following surgery and radiotherapy for breast cancer. Ann Surg Oncol. 2013;20:1267-1274.
  3. Manner J, Radlwimmer B, Hohenberger P, et al. MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema. Am J Pathol. 2010;176:34-39.
  4. Ginter PS, Mosquera JM, MacDonald TY, et al. Diagnostic utility of MYC amplification and anti-MYC immunohistochemistry in atypical vascular lesions, primary or radiation-induced mammary angiosarcomas, and primary angiosarcomas of other sites. Hum Pathol. 2014;45:709-716.
  5. Mentzel T, Schildhaus HU, Palmedo G, et al. Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological immunohistochemical and molecular analysis of 66 cases. Mod Pathol. 2012;25:75-85.
  6. Fernandez AP, Sun Y, Tubbs RR, et al. FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations. J Cutan Pathol. 2012;39:234-242.
  7. Morgan EA, Kozono DE, Wang Q, et al. Cutaneous radiation-associated angiosarcoma of the breast: poor prognosis in a rare secondary malignancy. Ann Surg Oncol. 2012;19:3801-3808.
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Dr. Arballo is from Brooke Army Medical Center, Fort Sam Houston, Texas. Drs. Beachkofsky and Kobayashi are from Wilford Hall Ambulatory Surgical Center, Lackland Air Force Base, Texas.

The authors report no conflict of interest.

The opinions expressed in this article are those of the authors and do not represent the viewpoints of the US Air Force, the US Army, or the Department of Defense.

Correspondence: Olivia M. Arballo, DO, Lake Erie College of Osteopathic Medicine, 1858 W Grandview Blvd, Erie, PA 16509 ([email protected]).

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Dr. Arballo is from Brooke Army Medical Center, Fort Sam Houston, Texas. Drs. Beachkofsky and Kobayashi are from Wilford Hall Ambulatory Surgical Center, Lackland Air Force Base, Texas.

The authors report no conflict of interest.

The opinions expressed in this article are those of the authors and do not represent the viewpoints of the US Air Force, the US Army, or the Department of Defense.

Correspondence: Olivia M. Arballo, DO, Lake Erie College of Osteopathic Medicine, 1858 W Grandview Blvd, Erie, PA 16509 ([email protected]).

Author and Disclosure Information

Dr. Arballo is from Brooke Army Medical Center, Fort Sam Houston, Texas. Drs. Beachkofsky and Kobayashi are from Wilford Hall Ambulatory Surgical Center, Lackland Air Force Base, Texas.

The authors report no conflict of interest.

The opinions expressed in this article are those of the authors and do not represent the viewpoints of the US Air Force, the US Army, or the Department of Defense.

Correspondence: Olivia M. Arballo, DO, Lake Erie College of Osteopathic Medicine, 1858 W Grandview Blvd, Erie, PA 16509 ([email protected]).

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The Diagnosis: Radiation-Associated Angiosarcoma

At the time of presentation, a 4-mm lesional punch biopsy was obtained (Figure), which revealed an epithelioid neoplasm within the dermis expressing CD31 and CD34, and staining negatively for S-100, CD45, and estrogen and progesterone receptors. The histologic and immunophenotypic findings were compatible with the diagnosis of angiosarcoma. Given the patient’s history of radiation for breast carcinoma several years ago, this tumor was consistent with radiation-associated angiosarcoma (RAAS).

Findings from a lesional punch biopsy were consistent with angiosarcoma (A and B)(H&E, original magnification ×40 and ×400, respectively).

Development of secondary angiosarcoma has been linked to both prior radiation (RAAS) and chronic lymphedema (Stewart-Treves syndrome).1 Radiation-associated angiosarcoma is defined as a “pathologically confirmed breast or chest wall angiosarcoma arising within a previously irradiated field.”2 The incidence of RAAS is estimated to be 0.9 per 1000 individuals following radiation treatment of breast cancer over the subsequent 15 years and a mean time from radiation to development of 7 years.1 Incidence is expected to increase in the future due to improved likelihood of surviving early-stage breast carcinoma and the increased use of external beam radiation therapy for management of breast cancer.

Differentiating between primary and secondary angiosarcoma of the breast is important. Although primary breast angiosarcoma usually arises in women aged 30 to 40 years, RAAS tends to arise in older women (mean age, 68 years) and is seen only in those women with prior radiation.2 Additionally, high-level amplification of MYC, a known photo-oncogene, on chromosome 8 is a key genetic alteration of RAAS that helps to distinguish it from primary angiosarcoma, though this variance may be present in only half of RAAS cases.3 Immunohistochemical analysis of tumor cells for MYC expression correlates well with this amplification and also is helpful in distinguishing atypical vascular lesions from RAAS.4 Atypical vascular lesions, similar to RAAS, occur years after radiation exposure and may have a similar clinical presentation. Atypical vascular lesions do not progress to angiosarcoma in reported cases, but clinical and histologic overlap with RAAS make the diagnosis difficult.5 In these cases, analysis with fluorescence in situ hybridization or immunohistochemistry for the MYC amplification is important to differentiate these tumors.6

At the time of presentation, the majority of patients with RAAS of the breast have localized disease, often with a variable presentation. In all known cases, there have been skin changes present, emphasizing the importance of both patient and clinician vigilance on a regular basis in at-risk individuals. In one study, the most common presentation was breast ecchymosis, which was observed in 55% of patients.7 These lesions involve the dermis and are commonly mistaken for benign conditions such as infection or hemorrhage.2 In 2 other studies, RAAS most often manifested as a skin nodule or apparent tumor, closely followed by either a rash or bruiselike presentation.1,2

The overall recommendation for management of patients with ecchymotic skin lesions in previously irradiated regions is to obtain a biopsy specimen for tissue diagnosis. Although there is no standard of care for the management of RAAS, a multidisciplinary approach involving specialists from oncology, surgical oncology, and radiation oncology is recommended. Most often, radical surgery encompassing both the breast parenchyma and the at-risk radiated skin is performed. Extensive surgery has demonstrated the best survival benefits compared to mastectomy alone.7 Chemotherapeutics also may be used as adjuncts to surgery, which have been determined to decrease local recurrence rates but have no proven survival benefits.2 Adverse prognostic factors for survival are tumor size greater than 10 cm and development of local and/or distant metastases.2 Following the diagnosis of RAAS, our patient underwent radical mastectomy with adjuvant chemotherapy and remained disease free 6 months after surgery.

In summary, RAAS is a well-known, albeit relatively uncommon, consequence of radiation therapy. Dermatologists, oncologists, and primary care providers play an important role in recognizing this entity when evaluating patients with ecchymotic lesions as well as nodules or tumors within an irradiated field. Biopsy should be obtained promptly to prevent delay in diagnosis and to expedite referral to appropriate specialists for further evaluation and treatment.

The Diagnosis: Radiation-Associated Angiosarcoma

At the time of presentation, a 4-mm lesional punch biopsy was obtained (Figure), which revealed an epithelioid neoplasm within the dermis expressing CD31 and CD34, and staining negatively for S-100, CD45, and estrogen and progesterone receptors. The histologic and immunophenotypic findings were compatible with the diagnosis of angiosarcoma. Given the patient’s history of radiation for breast carcinoma several years ago, this tumor was consistent with radiation-associated angiosarcoma (RAAS).

Findings from a lesional punch biopsy were consistent with angiosarcoma (A and B)(H&E, original magnification ×40 and ×400, respectively).

Development of secondary angiosarcoma has been linked to both prior radiation (RAAS) and chronic lymphedema (Stewart-Treves syndrome).1 Radiation-associated angiosarcoma is defined as a “pathologically confirmed breast or chest wall angiosarcoma arising within a previously irradiated field.”2 The incidence of RAAS is estimated to be 0.9 per 1000 individuals following radiation treatment of breast cancer over the subsequent 15 years and a mean time from radiation to development of 7 years.1 Incidence is expected to increase in the future due to improved likelihood of surviving early-stage breast carcinoma and the increased use of external beam radiation therapy for management of breast cancer.

Differentiating between primary and secondary angiosarcoma of the breast is important. Although primary breast angiosarcoma usually arises in women aged 30 to 40 years, RAAS tends to arise in older women (mean age, 68 years) and is seen only in those women with prior radiation.2 Additionally, high-level amplification of MYC, a known photo-oncogene, on chromosome 8 is a key genetic alteration of RAAS that helps to distinguish it from primary angiosarcoma, though this variance may be present in only half of RAAS cases.3 Immunohistochemical analysis of tumor cells for MYC expression correlates well with this amplification and also is helpful in distinguishing atypical vascular lesions from RAAS.4 Atypical vascular lesions, similar to RAAS, occur years after radiation exposure and may have a similar clinical presentation. Atypical vascular lesions do not progress to angiosarcoma in reported cases, but clinical and histologic overlap with RAAS make the diagnosis difficult.5 In these cases, analysis with fluorescence in situ hybridization or immunohistochemistry for the MYC amplification is important to differentiate these tumors.6

At the time of presentation, the majority of patients with RAAS of the breast have localized disease, often with a variable presentation. In all known cases, there have been skin changes present, emphasizing the importance of both patient and clinician vigilance on a regular basis in at-risk individuals. In one study, the most common presentation was breast ecchymosis, which was observed in 55% of patients.7 These lesions involve the dermis and are commonly mistaken for benign conditions such as infection or hemorrhage.2 In 2 other studies, RAAS most often manifested as a skin nodule or apparent tumor, closely followed by either a rash or bruiselike presentation.1,2

The overall recommendation for management of patients with ecchymotic skin lesions in previously irradiated regions is to obtain a biopsy specimen for tissue diagnosis. Although there is no standard of care for the management of RAAS, a multidisciplinary approach involving specialists from oncology, surgical oncology, and radiation oncology is recommended. Most often, radical surgery encompassing both the breast parenchyma and the at-risk radiated skin is performed. Extensive surgery has demonstrated the best survival benefits compared to mastectomy alone.7 Chemotherapeutics also may be used as adjuncts to surgery, which have been determined to decrease local recurrence rates but have no proven survival benefits.2 Adverse prognostic factors for survival are tumor size greater than 10 cm and development of local and/or distant metastases.2 Following the diagnosis of RAAS, our patient underwent radical mastectomy with adjuvant chemotherapy and remained disease free 6 months after surgery.

In summary, RAAS is a well-known, albeit relatively uncommon, consequence of radiation therapy. Dermatologists, oncologists, and primary care providers play an important role in recognizing this entity when evaluating patients with ecchymotic lesions as well as nodules or tumors within an irradiated field. Biopsy should be obtained promptly to prevent delay in diagnosis and to expedite referral to appropriate specialists for further evaluation and treatment.

References
  1. Seinen JM, Emelie S, Verstappen V, et al. Radiation-associated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection. Ann Surg Oncol. 2012;19:2700-2706.
  2. Torres KE, Ravi V, Kin K, et al. Long-term outcomes in patients with radiation-associated angiosarcomas of the breast following surgery and radiotherapy for breast cancer. Ann Surg Oncol. 2013;20:1267-1274.
  3. Manner J, Radlwimmer B, Hohenberger P, et al. MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema. Am J Pathol. 2010;176:34-39.
  4. Ginter PS, Mosquera JM, MacDonald TY, et al. Diagnostic utility of MYC amplification and anti-MYC immunohistochemistry in atypical vascular lesions, primary or radiation-induced mammary angiosarcomas, and primary angiosarcomas of other sites. Hum Pathol. 2014;45:709-716.
  5. Mentzel T, Schildhaus HU, Palmedo G, et al. Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological immunohistochemical and molecular analysis of 66 cases. Mod Pathol. 2012;25:75-85.
  6. Fernandez AP, Sun Y, Tubbs RR, et al. FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations. J Cutan Pathol. 2012;39:234-242.
  7. Morgan EA, Kozono DE, Wang Q, et al. Cutaneous radiation-associated angiosarcoma of the breast: poor prognosis in a rare secondary malignancy. Ann Surg Oncol. 2012;19:3801-3808.
References
  1. Seinen JM, Emelie S, Verstappen V, et al. Radiation-associated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection. Ann Surg Oncol. 2012;19:2700-2706.
  2. Torres KE, Ravi V, Kin K, et al. Long-term outcomes in patients with radiation-associated angiosarcomas of the breast following surgery and radiotherapy for breast cancer. Ann Surg Oncol. 2013;20:1267-1274.
  3. Manner J, Radlwimmer B, Hohenberger P, et al. MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema. Am J Pathol. 2010;176:34-39.
  4. Ginter PS, Mosquera JM, MacDonald TY, et al. Diagnostic utility of MYC amplification and anti-MYC immunohistochemistry in atypical vascular lesions, primary or radiation-induced mammary angiosarcomas, and primary angiosarcomas of other sites. Hum Pathol. 2014;45:709-716.
  5. Mentzel T, Schildhaus HU, Palmedo G, et al. Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological immunohistochemical and molecular analysis of 66 cases. Mod Pathol. 2012;25:75-85.
  6. Fernandez AP, Sun Y, Tubbs RR, et al. FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations. J Cutan Pathol. 2012;39:234-242.
  7. Morgan EA, Kozono DE, Wang Q, et al. Cutaneous radiation-associated angiosarcoma of the breast: poor prognosis in a rare secondary malignancy. Ann Surg Oncol. 2012;19:3801-3808.
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A 75-year-old woman with a history of stage II invasive ductal carcinoma of the right breast presented to the dermatology clinic with an enlarging, indurated, ecchymotic plaque on the inferior aspect of the right breast of 2 months’ duration. The patient underwent a lumpectomy, radiation, and adjuvant chemotherapy 13 years prior to presentation. Review of systems was otherwise noncontributory.

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